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Merck
CN

DMD

dystrophin

别名:
BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272
物种:
Human DMD (1756), Mouse Dmd (13405), Rat Dmd (24907), dog DMD (606758), chicken DMD (396236), Zebrafish dmd (83773), cow DMD (540755), Horse DMD (100051515), domestic cat DMD (100750243), sheep DMD (101119444) ...展开
UniProtKB ID:
P11532, E9PDN1, Q02295, Q14169, Q14170, Q5JYU0, Q6NSJ9, Q7KZ48
基因ID:
  • Human(1756) Summary: The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. [provided by RefSeq, Jul 2008]
  • Mouse(13405) dystrophin, muscular dystrophy
  • Rat(24907) dystrophin
  • dog(606758) dystrophin
  • chicken(396236) dystrophin
  • Zebrafish(83773) dystrophin
  • cow(540755) dystrophin
  • Horse(100051515) dystrophin
  • domestic cat(100750243) Duchenne muscular dystrophy
  • sheep(101119444) dystrophin
  • naked mole-rat(101715920) dystrophin
  • domestic guinea pig(100727002) dystrophin

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Antibodies

货号
说明
物种反应性
应用
MABT827
Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), clone 2C6 (MANDYS106), from mouse,
物种反应性
human
应用
immunofluorescence
immunohistochemistry
western blot
SAB4200764
Anti-Dystrophin antibody, Mouse monoclonal, clone MANDYS8, purified from hybridoma cell culture,
物种反应性
human, mouse, porcine, rabbit, rat
应用
immunohistochemistry
western blot
SAB4200763
Anti-Dystrophin antibody, Mouse monoclonal, clone MANDRA1, purified from hybridoma cell culture,
物种反应性
human, mouse, rat, zebrafish
应用
immunofluorescence
immunohistochemistry
western blot
ZRB1309
Anti-Dystrophin Antibody, clone 1G7, ZooMAb® Rabbit Monoclonal, recombinant, expressed in HEK 293 cells,
物种反应性
human, mouse
应用
ELISA
affinity binding assay
immunocytochemistry
immunohistochemistry
western blot
MABT827-AF488
Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor™ 488 Conjugate, clone 2C6 (MANDYS106), from mouse, ALEXA FLUOR™ 488,
物种反应性
human
应用
immunofluorescence